Ewing's sarcoma family of tumor arising in the adrenal gland—Possible diagnostic pitfall in pediatric pathology: Histologic, immunohistochemical, ultrastructural, and molecular study

doi:10.1053/hupa.2001.27121

Human Pathology

Volume 32, Issue 9, September 2001, Pages 1012-1016

https://doi.org/10.1053/hupa.2001.27121 Get rights and content

Abstract

We present an adrenal Ewing's sarcoma family of tumor (ESFT) arising in an 11-year-old Japanese boy. Although intensive chemoradiotherapy and radical surgery were performed, the patient died of obstinate disease 1 year and 3 months after the initial presentation. The primary site (adrenal gland) with radiologic findings (with foci of calcification), high titer of serum neuron specific enolase, and sheets of monotonous primitive rounded cells on histology mostly favored neuroblastoma. However, a diagnosis of ESFT was confirmed by immunohistochemical profile, including MIC2-positivity and molecular study disclosing EWS-FLI1 chimera gene verified by direct sequencing. Recognition of adrenal ESFT and use of newly developed diagnostic techniques are required for differential diagnosis of undifferentiated small round cell tumor of the adrenal gland. HUM PATHOL 32:1012-1016. Copyright © 2001 by W.B. Saunders Company

Section snippets

Case report

An 11-year-old boy was referred to our institution because of a right upper quadrant abdominal tumor that had been noted for about 2 weeks. His past and family history was not contributory. Physical examination showed an elastic hard tumor on the right upper quadrant abdomen. A multinodular tumor with heterogeneous internal density and foci of calcification was identified in the right suprarenal region on computed tomography. No distant metastatic lesion was identified on systemic radiologic

Routine histopathology

The tumor tissues were fixed in 10% buffered formalin and embedded in paraffin wax. Four-micron-thick sections were stained with hematoxylin and eosin after dewaxing. The sections were also stained with periodic acid-Schiff (PAS) with or without diastase predigestion.

Immunohistochemistry

Immunohistochemistry on paraffin-embedded sections by indirect immunoperoxidase method was performed by using antibodies against vimentin, cytokeratin (clone CAM5.2), desmin, neurofilament, muscle actin, CD99 (MIC2, clone 12E7),

Histopathologic findings

The biopsy sample showed sheets of neoplastic cells intermingled with fibrovascular stroma and necrotic areas. The neoplastic cells had ill-defined scanty cytoplasm and hyperchromatic round-to-oval vesicular nuclei with 1 or 2 distinct eosinophilic nucleoli (Fig 1).

. Histology of the biopsy sample that shows monotonous growth of neoplastic cells having scanty and ill-defined cytoplasm and vesicular round-to-oval nuclei with distinct nucleoli.

Mitotic activity was frequently seen (5-7 per

Discussion

ESFTs usually occur in bone and soft tissue.1, 2 However, other primary sites such as the kidney, lung, or subcutaneous tissue have been described recently.³ About 25% of the ESFT cases develop at distant sites such as the lung, bone, and bone marrow at initial diagnosis.1, 2 However, metastasis to the adrenal gland is quite exceptional.⁹ The present patient had no apparent evidence of a primary site other than the adrenal gland. On histology, a rim of the adrenal cortex surrounded most of the

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Adrenal mass of unusual etiology: Ewing sarcoma in a young man
2017, Radiology Case Reports
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In 4 patients, venous thrombus was detected and was removed by thrombectomy. As of 2013, 3 patients showed no evidence of disease on a mean 12-month follow-up, whereas 4 patients were alive with disease (Table 1) [1–4,6,9,14,29–32]. As a summary, according to original reports, 6 of these 19 cases died—1 was a suicidal patient, and 5 had metastases.
Ewing sarcoma and peripheral primitive neuroectodermal tumor belong to the Ewing sarcoma (ES) family of tumors originating from a primitive neural tube. We report a 31-year-old man who was admitted to the urology clinic with complaints of fever, nausea, and dysuria. A right-sided adrenal mass was detected during ultrasonography. The lesion was then evaluated with magnetic resonance imaging, which showed areas of necrosis amid heterogeneous solid areas. Whole body scan with 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography and bone scan studies showed pulmonary and osseous metastatic foci. The mass and right kidney were removed by an open approach. An immunohistochemical and molecular workup enabled the diagnosis of ES. The patient also underwent radiotherapy and chemotherapy. The patient remained in fairly good health during the 18-month follow-up period, but showed progression of all metastatic foci and died 26 months after treatment. In conclusion, adrenal ES should be included in the differential diagnosis of nonfunctional adrenal lesions despite its rare occurrence.
Latent adrenal Ewing sarcoma family of tumors: A case report
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Ewing sarcoma family of tumors (ESFT) is derived from the neural crest, which originates from basal embryo cells in the primitive neural tube. ESFT often arises at the bone, chest wall, and soft tissues of the thoracic region. However, ESFT that arises from the adrenal gland is much rarer and it is usually revealed by clinical symptoms. We report an autopsy case of suicidal hanging, in which adrenal ESFT was incidentally revealed. To our knowledge, this is the first case of latent ESFT arising from the adrenal gland. Autopsy can sometimes reveal latent disease. Some of these latent diseases are very rare and we would not be able to detect them without a complete autopsy. As forensic pathologists, we should attempt to perform a complete autopsy and report new discoveries for the development of medicine.
An unusual adrenal tumor: Ewing tumor
2011, Annales de Pathologie
Nous rapportons le cas d’une volumineuse tumeur de la loge surrénalienne, découverte chez une jeune femme enceinte à 26 semaines d’aménorrhée. Morphologiquement, cette tumeur molle, blanchâtre avec des secteurs hémorragiques, était constituée de plages de cellules de taille moyenne, monomorphes, polygonales ou fusiformes, avec de nombreuses mitoses. Les cellules tumorales exprimaient les pancytokératines AE1/AE3, l’epithelial membrane antigen (EMA), la vimentine et le CD99. Les diagnostics évoqués étaient ceux de synovialosarcome peu différencié, de carcinome sarcomatoïde et de tumeur d’Ewing. Grâce à la biologie moléculaire, mettant en évidence un transcrit spécifique des tumeurs de la famille Ewing/peripheral primitive neuroectodermal tumor (pPNET), le transcrit EWS/FLI1, le diagnostic de certitude de cette tumeur d’aspect atypique et de localisation inhabituelle a été établi. En effet, 75 % des tumeurs d’Ewing sont de siège osseux, avec une prédilection pour la diaphyse des os longs et 20 à 25 % s’observent dans les tissus mous et la peau. Les localisations primitives viscérales sont rares ; moins d’une dizaine de cas de localisation surrénalienne a été rapporté. La localisation intrasurrénalienne inhabituelle de cette tumeur pourrait être liée au fait que les cellules souches mésenchymateuses, à l’origine de cette tumeur, dériveraient éventuellement des cellules des crêtes neurales. Le diagnostic de tumeur d’Ewing repose sur un faisceau d’arguments histopathologiques et de biologie moléculaire, a fortiori dans les cas morphologiques atypiques.
We report the case of a voluminous tumor of the adrenal diagnosed in a young pregnant woman at 26^th week of amenorrhea. Morphologically, a soft white tumor with haemorragic areas was observed, made of sheets of monomorphous, medium sized, spindle-shaped to polygonal, with high mitotic activity. Tumorous cells expressed cytokeratins AE1/AE3, EMA, and CD99 (expression of vimentin is not relevant). Contemplated diagnoses included poorly differentiated synovialosarcoma, sarcomatoid carcinoma and Ewing tumor. Thanks to molecular biology, showing the specific transcript of Ewing/peripheral primitive neuroectodermal tumor (pPNET) EWS/FLI1, the diagnosis of this atypical tumor in an unusual location was performed. Indeed, 75% of Ewing tumors involve bones (especially, the diaphysis of long bones) and 20 to 25% soft tissues. Primitive visceral involvement is rare; less than 10 cases of adrenal involvement have been reported. The hypothesis that Ewing cell's origin is a mesenchymal stem cell, which may derive from neural crest cell, could explain the uncommon adrenal involvement. Diagnosis of Ewing tumor is based on pathologic and molecular findings, especially in atypical cases.
Anaplastic sarcoma of the kidney with chromosomal abnormality: First report on cytogenetic findings
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Immunohistochemical study was performed using antibodies against the antigens, as shown in Table 1, and the indirect immunoperoxidase method. Frozen section tumor samples were studied for EWS-FLI1, EWSR1-ERG, EWSR1-WT1, ETV6-NTRK3, SS18-SSX, PAX3-FOXO1A, and PAX7-FOXO1A chimeric transcripts, as described previously [3-7]. Chromosomal preparations of disaggregated tumor cells were made after 3 days culture using standard methods.
We report a case of anaplastic sarcoma of the kidney (ASK) with cytogenetic findings. A 12-year-old Japanese girl presented with buttock pain and urinary incontinence. Radiological investigations revealed a right renal tumor with multiple distant metastases and multicystic thyroid tumor. She underwent radical right nephrectomy and subsequently received chemotherapy and radiation therapy. Histologically, the renal tumor demonstrated admixture of various types of mesenchymal elements: cellular spindle cells with anaplastic features, cartilage, and rhabdomyoblastic cells consistent with ASK. Chromosomal analysis revealed the karyotype of the tumor cells to be 46, XX, +8, –10, der (18) t (10; 18) (q21; p11.2). The thyroid tumor was removed later and diagnosed as adenomatous goiter. To our knowledge, this is the first case of ASK with chromosomal abnormality and may provide new insight into the molecular biologic basis of this rare renal tumor.
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^*: Supported in part by Kanagawa Children's Medical Investigation Fund.

^**: Address correspondence and reprint requests to Keisuke Kato, MD, Division of Pathology, Kanagawa Children's Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama, 232-8555, Japan.

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Case StudiesEwing's sarcoma family of tumor arising in the adrenal gland—Possible diagnostic pitfall in pediatric pathology: Histologic, immunohistochemical, ultrastructural, and molecular study*,**

Abstract

Section snippets

Case report

Routine histopathology

Immunohistochemistry

Histopathologic findings

Discussion

Hum Pathol

J Urol

J Biol Chem

Eur J Cancer

J Pediatr Surg

Hum Pathol

Eur J Cancer

Case Studies
Ewing's sarcoma family of tumor arising in the adrenal gland—Possible diagnostic pitfall in pediatric pathology: Histologic, immunohistochemical, ultrastructural, and molecular study*,**